Livedo Racemosa - The Pathophysiology of Decompression-Associated Cutis Marmorata and Right/Left Shunt.

Decompression sickness and arterial gas embolism, collectively known as decompression illness (DCI), are serious medical conditions that can result from compressed gas diving. DCI can present with a wide range of physiologic and neurologic symptoms. In diving medicine, skin manifestations are usually described in general as cutis marmorata (CM). Mainly in the Anglo-American literature the terms cutis marmorata, livedo reticularis (LR), and livedo racemosa (LRC) are used interchangeably but actually describe pathophysiologically different phenomena. CM is a synonym for LR, which is a physiological and benign, livid circular discoloration with a net-like, symmetric, reversible, and uniform pattern. The decompression-associated skin discolorations, however, correspond to the pathological, irregular, broken netlike pattern of LRC. Unlike in diving medicine, in clinical medicine/dermatology the pathology of livedo racemosa is well described as a thrombotic/embolic occlusion of arteries. This concept of arterial occlusion suggests that the decompression-associated livedo racemosa may be also caused by arterial gas embolism. Recent studies have shown a high correlation of cardiac right/left (R/L) shunts with arterial gas embolism and skin bends in divers with unexplained DCI. To further investigate this hypothesis, a retrospective analysis was undertaken in a population of Austrian, Swiss, and German divers. The R/L shunt screening results of 18 divers who suffered from an unexplained decompression illness (DCI) and presented with livedo racemosa were retrospectively analyzed. All of the divers were diagnosed with a R/L shunt, 83% with a cardiac shunt [patent foramen ovale (PFO)/atrium septum defect (ASD)], and 17% with a non-cardiac shunt. We therefore not only confirm this hypothesis but when using appropriate echocardiographic techniques even found a 100% match between skin lesions and R/L shunt. In conclusion, in diving medicine the term cutis marmorata/livedo reticularis is used incorrectly for describing the actual pathology of livedo racemosa. Moreover, this pathology could be a good explanation for the high correlation of livedo racemosa with cardiac and non-cardiac right/left shunts in divers without omission of decompression procedures.

Augmentation in restless legs syndrome: an eye tracking study on emotion processing.

OBJECTIVE: To assess emotional processing and alexithymia in patients with restless legs syndrome (RLS) with augmentation versus those who never had augmentation. METHODS: We recruited 26 patients who had a history of augmentation (AUG), either current or past, 27 RLS patients treated with dopamine agonists who never had augmentation (RLS controls), and 21 healthy controls (HC). All participants were screened for impulse control disorders (ICDs). Alexithymia was assessed by means of the Toronto Alexithymia Scale - 20 (TAS-20). Facial emotion recognition was tested through an eye-tracking task. Furthermore, all participants performed neuropsychological tests assessing global cognitive status, impulsivity, anxiety, and depression. RESULTS: ICD symptoms occurred more frequently in AUG patients than in RLS controls (P = 0.047). Patients with AUG scored higher on the TAS-20 (P = 0.007) and the attentional subdomain of an impulsivity scale (BIS-11; P = 0.015) compared to HC. Patients with AUG also performed worse on the facial emotion recognition task relative to RLS controls (P = 0.009) and HC (P = 0.003). We found a group difference for the time to first fixation and the fixation count in the mouth region (P = 0.019 and P = 0.021, respectively). There were no other differences in the eye tracking examination. INTERPRETATION: This study showed evidence of poorer emotional processing in patients who had augmentation compared to RLS patients without augmentation and healthy controls. The altered exploration pattern of faces and the higher alexithymia scores suggest abnormalities in emotion processing in patients with augmentation.

Eye-Tracking Provides a Sensitive Measure of Exploration Deficits After Acute Right MCA Stroke.

The eye-tracking study aimed at assessing spatial biases in visual exploration in patients after acute right MCA (middle cerebral artery) stroke. Patients affected by unilateral neglect show less functional recovery and experience severe difficulties in everyday life. Thus, accurate diagnosis is essential, and specific treatment is required. Early assessment is of high importance as rehabilitative interventions are more effective when applied soon after stroke. Previous research has shown that deficits may be overlooked when classical paper-and-pencil tasks are used for diagnosis. Conversely, eye-tracking allows direct monitoring of visual exploration patterns. We hypothesized that the analysis of eye-tracking provides more sensitive measures for spatial exploration deficits after right middle cerebral artery stroke. Twenty-two patients with right MCA stroke (median 5 days after stroke) and 28 healthy controls were included. Lesions were confirmed by MRI/CCT. Groups performed comparably in the Mini-Mental State Examination (patients and controls median 29) and in a screening of executive functions. Eleven patients scored at ceiling in neglect screening tasks, 11 showed minimal to severe signs of unilateral visual neglect. An overlap plot based on MRI and CCT imaging showed lesions in the temporo-parieto-frontal cortex, basal ganglia, and adjacent white matter tracts. Visual exploration was evaluated in two eye-tracking tasks, one assessing free visual exploration of photographs, the other visual search using symbols and letters. An index of fixation asymmetries proved to be a sensitive measure of spatial exploration deficits. Both patient groups showed a marked exploration bias to the right when looking at complex photographs. A single case analysis confirmed that also most of those patients who showed no neglect in screening tasks performed outside the range of controls in free exploration. The analysis of patients' scoring at ceiling in neglect screening tasks is of special interest, as possible deficits may be overlooked and thus remain untreated. Our findings are in line with other studies suggesting considerable limitations of laboratory screening procedures to fully appreciate the occurrence of neglect symptoms. Future investigations are needed to explore the predictive value of the eye-tracking index and its validity in everyday situations.

Continuous intra-arterial nimodipine infusion in refractory symptomatic vasospasm after subarachnoid hemorrhage.

INTRODUCTION: Vasospasm still is a major cause of morbidity after aneurysmal subarachnoid hemorrhage. The purpose of this report is to describe the successful management of severe refractory vasospasm with continuous intra-arterial nimodipine (IAN) treatment. CASE DESCRIPTION: A 72-year old right handed woman was admitted with non-traumatic SAH WFNS grade 1. Cerebral computed tomography demonstrated thick blood filling of the basal cisterns, and intraventricular hemorrhage. Cerebral angiogram failed to detect a vascular abnormality. After an uneventful initial course the patient developed symptomatic left middle cerebral artery vasospasm with aphasia and corresponding restriction in diffusion weighted images in the left frontal lobe. Bolus IAN only transiently improved cerebral circulation and clinical signs and symptoms. Continuous-IAN was started and led to full clinical recovery and normalisation of MRI diffusion restrictions. DISCUSSION AND CONCLUSIONS: Continuous selective intra-arterial infusion of nimodipine may be an option in selected patients with symptomatic vasospasm refractory to conventional treatment after careful consideration of benefits and procedure-related risks.

Substantia nigra hyperechogenicity and Parkinson's disease risk in patients with essential tremor.

BACKGROUND: Several studies have reported an increased risk for patients with essential tremor to develop Parkinson's disease. In addition, hyperechogenicity in the area of the substantia nigra has been associated with a markedly increased risk for Parkinson's disease. The objective of this study was to evaluate the validity of substantia nigra hyperechogenicity in patients with essential tremor as a risk marker for Parkinson's disease. METHODS: Transcranial sonography was performed in 70 patients suffering from essential tremor. Fifty-four of these patients were available for follow-up after a mean of 6.16 ± 2.05 years and were assessed for the incidence of new-onset Parkinson's disease. RESULTS: The relative risk for developing Parkinson's disease in patients with essential tremor who had hyperechogenicity at baseline versus those without this hyperechogenicity was 7.00 (95% confidence interval, 1.62-30.34; sensitivity, 77.8%; specificity, 75.6%). CONCLUSIONS: Substantia nigra hyperechogenicity is also associated with an increased risk for Parkinson's disease in patients with essential tremor. These findings further support the potential role of this echofeature as a risk marker for Parkinson's disease.

Meralgia paraesthetica: Ultrasound-guided injection at multiple levels with 12-month follow-up.

OBJECTIVES: To evaluate the efficacy of ultrasound (US)-guided injections around the lateral femoral cutaneous nerve (LFCN) at different levels in meralgia paraesthetica (MP) patients. METHODS: The study was approved by the university ethics committee and informed oral and written consent were obtained from all patients. Between June 2008 and August 2013, 20 patients with symptoms of MP, including nine men (mean age, 61.33 years) and 11 women (mean age 61.18 years), were treated with US-guided injection of steroids along the LFCN at three different levels in a mean of 2.25 sessions. A visual analogue scale (VAS) was used to measure symptoms before, immediately after and 12 months after treatment. RESULTS: Complete resolution of symptoms was documented in 15/20 patients (mean VAS decreased from 82 to 0), and partial resolution in the remaining five (mean VAS decreased from 92 to 42), which was confirmed at 12-month follow-up. By using the different levels of injection approach overall significantly better symptom relief was obtained (p < 0.05). CONCLUSION: The outcome of US-guided injection along the LFCN can be further improved by injections at different levels (p < 0.05), which was confirmed at 12-month long-term follow-up. KEY POINTS: Meralgia paraesthetica is an entrapment neuropathy of the lateral femoral cutaneous nerve. Ultrasound proved effective in diagnosis and in guiding injection therapy. Injection at the anterior superior iliac spine has been used previously. Multiple injections along the nerve course were used in this study. Long-term follow-up (12 months) confirmed the results.

Sonographic cross-sectional area measurement in carpal tunnel syndrome patients: can delta and ratio calculations predict severity compared to nerve conduction studies?

OBJECTIVE: To evaluate the accuracy of two different sonographic median nerve measurement calculations in predicting carpal tunnel syndrome (CTS) severity in a study population with clinically and electrophysiologically confirmed CTS. METHODS: 643 wrists of 427 patients (325 females and 102 males, age range: 17-90 years, mean ± SD: 57.9 ± 14.7) were included with CTS diagnosis based on clinical and nerve conduction studies (NCS). Cross-sectional area (CSA) measurement of the median nerve was performed at the carpal tunnel level (CSAc) and at the pronator quadratus muscle level (CSAp). Two parameters were calculated: delta (∆-CSA), which is the difference between proximal and distal measurements, and ratio (R-CSA), calculated by dividing distal over proximal measurements. RESULTS: Patients were classified into mild, moderate and severe CTS based upon NCS. The mean ∆-CSA (4.2 ± 2.6, 6.95 ± 2.2 and 10.7 ± 4.9 mm(2)) and mean R-CSA (1.5 ± 0.4, 1.95 ± 0.4 and 2.4 ± 0.7) values were significantly different between all groups (p < 0.001). Optimal cut-off values for ∆-CSA and R-CSA were 6 mm(2) and 1.7, respectively, to distinguish mild from moderate disease, and 9 mm(2) and 2.2, respectively, to distinguish moderate from severe disease. CONCLUSION: Threshold values for the calculated sonographic parameters ∆-CSA and R-CSA are useful in predicting CTS severity compared to NCS. KEY POINTS: • Two proposed parameters were calculated (∆-CSA, R-CSA) and compared to NCS. • A defined sonoanatomical proximal landmark was used for the calculation. • Both parameters showed ability to detect CTS severity comparable to NCS. • Cut-off values could be determined for both parameters.

Enhancement patterns in the fibro cellular tissue in different kinds of plaques of the internal carotid artery.

BACKGROUND: The differentiation between stable and vulnerable plaques in the internal carotid artery (ICA) remains a matter of interest. With the implementation of contrast agent in magnetic resonance imaging (MRI) a more detailed plaque characterization is possible. The study at hand focuses on enhancement patterns of fibro cellular tissue in different kinds of plaques in the ICA. METHODS: Between May 2011 and December 2012, 49 patients (39 male) with >50% stenosis of the ICA were consecutively enrolled. In 10 patients with bilateral ICA stenosis, both plaques were included for analysis. We performed a classification of plaques according to Cai and observed 11 type 4-5 plaques, 15 type 6 plaques and 33 type 8 plaques. MRI was performed on a 3T whole body MR system. The standard 12 channel head coil was combined with the neck extension coil and two bilateral 7 cm loop coils. Post-contrast T1w images were subtracted from pre contrast images to identify late enhancement in fibro cellular tissue. Enhancement patterns were allocated as intraluminal, intraplaque and vasa vasorum enhancement in different types of plaques. RESULTS: Fibro cellular tissue always exhibited a higher contrast enhancement compared to the sternocleidomastoid muscle. This reflects a higher grade of vascularization of the fibrocellular tissue. Contrast enhancement was present irrespective of the plaque type. In detail, intraluminal, intraplaque and vasa vasorum enhancement were observed in all types of plaques. Even type 8 plaques, according to the classification of Cai, had a significant contrast enhancement, though supposed to be with low inflammatory activity. CONCLUSION: Type 8 plaques might not be as stable as postulated. Whether the relevant uptake of contrast agent is due to the fibrous tissue or reflects the inflammatory activity of the plaque should be matter of further investigations.

High dose erythropoietin increases brain tissue oxygen tension in severe vasospasm after subarachnoid hemorrhage.

BACKGROUND: Vasospasm-related delayed cerebral ischemia (DCI) significantly impacts on outcome after aneurysmal subarachnoid hemorrhage (SAH). Erythropoietin (EPO) may reduce the severity of cerebral vasospasm and improve outcome, however, underlying mechanisms are incompletely understood. In this study, the authors aimed to investigate the effect of EPO on cerebral metabolism and brain tissue oxygen tension (PbtO2). METHODS: Seven consecutive poor grade SAH patients with multimodal neuromonitoring (MM) received systemic EPO therapy (30.000 IU per day for 3 consecutive days) for severe cerebral vasospasm. Cerebral perfusion pressure (CPP), mean arterial blood pressure (MAP), intracranial pressure (ICP), PbtO2 and brain metabolic changes were analyzed during the next 24 hours after each dose given. Statistical analysis was performed with a mixed effects model. RESULTS: A total of 22 interventions were analyzed. Median age was 47 years (32-68) and 86 % were female. Three patients (38 %) developed DCI. MAP decreased 2 hours after intervention (P < 0.04) without significantly affecting CPP and ICP. PbtO2 significantly increased over time (P < 0.05) to a maximum of 7 ± 4 mmHg increase 16 hours after infusion. Brain metabolic parameters did not change over time. CONCLUSIONS: EPO increases PbtO2 in poor grade SAH patients with severe cerebral vasospasm. The effect on outcome needs further investigation.

Bifid median nerve in carpal tunnel syndrome: assessment with US cross-sectional area measurement.

PURPOSE: To evaluate the accuracy of ultrasonography (US) in the diagnosis of carpal tunnel syndrome (CTS) in patients with a bifid median nerve on the basis of cross-sectional area (CSA) measurements of the median nerve at the level of the carpal tunnel (CSAc), with additional measurements obtained more proximally (CSAp) at the level of the pronator quadratus muscle. MATERIALS AND METHODS: This HIPAA-compliant study was approved by the local institutional review board; informed oral and written consent were obtained. Fifty-three wrists in 49 consecutive patients with a bifid median nerve and CTS symptoms and 28 wrists in 27 healthy volunteers with a bifid median nerve were examined by using US. Two independent US examiners who were blinded to prior test results measured median nerve CSA at two levels, CSAc and CSAp. The difference between CSAc and CSAp (ΔCSA) was calculated for each wrist. Receiver operating characteristic (ROC) analysis was performed. RESULTS: The study population included 17 men and 32 women (mean age, 55.1 years; age range, 24-78 years). The control population included 13 men and 14 women (mean age, 52.6 years; age range, 24-86 years). Mean CSAc was approximately 5 mm(2) greater in patients with CTS than in healthy volunteers (P < .0001), while mean ΔCSA was 5.8-5.9 mm(2) greater in patients with CTS (P < .0001). A CSAc threshold of 12 mm(2) provided sensitivity and specificity of 84.9% and 46.5%, respectively, while a ΔCSA threshold of 4 mm(2) provided sensitivity and specificity of 92.5% and 94.6%, respectively. ROC analysis demonstrated a significant advantage of ΔCSA (area under ROC curve [A(z)] = 0.95-0.96) compared with CSAc (A(z) = 0.84-0.85) for the diagnosis of CTS (P < .003). CONCLUSION: The use of a ΔCSA parameter improves the diagnostic accuracy of US for the presence of CTS in patients with a bifid median nerve.

Multimodal neuromonitoring in a patient with aneurysmal subarachnoid hemorrhage associated with aortic coarctation.

BACKGROUND: Aortic coarctation (AC) rarely remains undiagnosed until adulthood. Intracranial aneurysms and spontaneous subarachnoid hemorrhage (SAH) are more frequent in patients with AC than in the general population. METHODS: The purpose of this report is to describe the management of a poor grade SAH patient with previously undiagnosed AC using advanced monitoring techniques of the brain and the cardiovascular system. RESULTS: A 28-year-old man with previously unknown AC was admitted with aneurysmal SAH WFNS grade 5. Head computed tomography demonstrated thick blood filling the basal cisterns, mild hydrocephalus, and global cerebral edema. The ruptured aneurysm of the anterior communicating artery was successfully coiled on the same day. Echocardiography revealed high grade isthmus stenosis with a pressure gradient of 60 mm Hg. Hospital course was complicated by prerenal failure and severe vasospasm. Neuromonitoring data (cerebral metabolism, brain tissue oxygenation, cerebral blood flow and intracranial pressure) were used as endpoint to define the optimal blood pressure to meet the cerebral metabolic and oxygen demand in each phase. CONCLUSIONS: Cerebral aneurysm rupture associated with coarctation of the aorta is rare. The aim of this report is to call attention to AC in, particularly, young patients presenting with SAH and to discuss advanced multimodal neuromonitoring techniques used to optimize intensive care management.

Transcranial sonography in restless legs syndrome.

Restless legs syndrome (RLS) is one of the most common neurological disorders. The diagnosis is mainly based on the patient's report of the characteristic symptoms and further on the response to dopaminergic treatment. In the past years, transcranial B-Mode sonography (TCS) of the mesencephalic brainstem has been introduced as a promising new method to assist the diagnostic approach to RLS. This review of literature describes the typical TCS findings in RLS including hypoechogenicity of the substantia nigra and raphe as well as hyperechogenicity of the red nucleus. The diagnostic parameters for patients with idiopathic and various forms of symptomatic RLS will be discussed as well as the current knowledge about the pathophysiology underlying these sonographical abnormalities.

Midbrain hyperechogenicity in idiopathic REM sleep behavior disorder.

Recent studies have reported an increased risk to develop Parkinson's disease (PD) in patients with idiopathic RBD (iRBD). Midbrain hyperechogenicity is a common transcranial sonography (TCS) finding in PD and has been suggested as a PD risk-marker in nonparkinsonian subjects. The objective of this study is to assess midbrain echogenicity by TCS in patients with iRBD and compare the findings with the healthy controls. TCS was performed in 55 iRBD patients and in 165 age and sex-matched controls. The area of echogenicity in the SN region in the iRBD group was significantly increased compared with the control group (P < 0.001). About 19 (37.3%) of patients with iRBD were found to have SN hyperechogenicity when compared with 16 (10.7%) of the controls (P < 0.001). This is the first case-control study assessing midbrain echogenicity in a large iRBD cohort compared to age- and sex-matched healthy individuals. The finding of an increased prevalence of hyperechogenicity in a subgroup of individuals with a priori increased risk for PD supports the potential role of hyperechogenicity as a risk marker for PD. The prospective follow-up of this iRBD cohort is needed to establish if those with midbrain hyperechogenicity will go on to develop clinically defined PD or not.

[Implementation of thrombolysis in acute stroke--10-year results of the Innsbruck stroke registry]. / Einsatz der Thrombolyse beim akut ischämischen Schlaganfall : 10-Jahres-Ergebnisse an der Stroke Unit Innsbruck.

BACKGROUND: Randomized controlled trials have yielded evidence for the efficacy and safety of intravenous alteplase in the therapy of acute ischemic stroke. A large patient registry has recently confirmed the safe implementation of this therapy in the clinical routine setting. METHODS: Between January 1998 and December 2007 302 stroke patients were treated with 0.9 mg/kg rt-PA at the stroke unit of the Innsbruck University Hospital. Severity and circumstances of the stroke event, indicators of pre- and intrahospital management as well as safety and outcome at three months were prospectively assessed in the local thrombolysis database. RESULTS: The number of patients receiving intravenous thrombolysis increased continuously from 2 patients in 1998 to 67 in 2007 and 87 patients in 2008. 43% of our patients were females. The median age and NIHSS-score on admission was 67 and 16, respectively. The mean onset-to-needle time decreased from 171 min to 110 min--mainly due to a substantial shortening of the door-to-needle time from 105 min to 45 min. A proportion of 41% of our patients were treated in the main working time while 59% received rt-PA during night and weekend service. A total of 38% of our patients were functionally independent at three months (mRS 0-2). Once considering the high initial stroke severity in our patient series and correcting the NIHSS scores to levels usually seen in randomized control trials and patient registries, 56% of our patients would reach a good outcome (mRS 0-2). The rate of symptomatic intracranial bleedings was low at 6.3%. CONCLUSION: Our data reinforce that intravenous thrombolysis is safe in the treatment of acute ischemic stroke in clinical routine setting. Establishment of modern stroke services and the implementation of structural operating procedures have contributed to an increase in the number of treated patients and a parallel decrease in door-to-needle time at our hospital. Widespread educational programs in the general community, introduction of optimized pre-hospital triage algorithms as well as the potential extension of the 3-hour window to 4.5 hours all are suitable measures to further extend the benefit of i.v. thrombolysis to large proportion of stroke patients.

Midbrain sonography in patients with essential tremor.

Several studies have reported an increased risk to develop Parkinson's disease (PD) in essential tremor (ET) populations. Hyperechogenicity of the substantia nigra (SN) is a common transcranial sonography (TCS) finding in PD and has been suggested as a PD risk-marker in nonparkinsonian subjects. This study compared SN areas of 44 ET patients with 100 controls and 100 PD patients. Sixteen percent of ET patients had SN hyperechogenicity as compared with 3% of controls and 75% of PD patients. These findings might correspond to an increased risk of ET patients to develop PD. Long-term follow-up will show if those with hyperechogenic SN develop PD, while these without will not.

Transcranial ultrasound shows nigral hypoechogenicity in restless legs syndrome.

In patients with Parkinson's disease, hyperechogenicity of the substantia nigra using transcranial ultrasound has been related to increased tissue concentrations of iron. Recently, deficient iron transport mechanisms in substantia nigra neurons have been described in postmortem tissue of patients with restless legs syndrome (RLS). This study was performed to study substantia nigra echogenicity in RLS patients compared with normal control subjects and Parkinson's disease patients. RLS patients had significantly reduced midbrain areas of hyperechogenicity compared with control subjects, and even more markedly reduced hyperechogenicity compared with Parkinson's disease patients. These findings lend further support to nigral iron deficiency as a pathogenetic factor in RLS.